Addisons disease

Primary

adrenocortical insufficiency is rare (incidence 0.8 per 100.000), 1tsigns are capricious: as diagnosis may only be made at necropsy its called the unforgiving master of non-specificity and disguise.

Causes
80% idiopathic in UK, probably autoimmune (adrenal antibodies raised). Associated with Graves, Hashimotos, IDDM, pernicious anaemia, hypoparathyroidism, vitiligo. Other causes: TB; metastases (adrenal insufficiency only after >90% involvement of both adrenals); AIDS (CMV Mycobacterium svium intracellulare, other opportunistic adrenal infections)

Symptoms
Weakness Depression Anorexia Arthralgia Weight loss Constipation Abdominal pain Viral illness D& v (or nausea) Myaigia Confusion Dizziness

Signs:
Hyperpigmentation (palmar creases, buccal mucosa), vitiligo, postural hypotension. Critical deterioration is indicated by tachycardia, fever, shock, and coma.

Tests:
General: K+ Na+ glucose uraemia, mild acidosis, Ca2+ eosinophilia, neutropenia. lymphocytosis. anaemia, abnormal LFTs,

Specific Short ACTH (stimulation test (Svnacthen test): Do plasma cortisol before and (1/2h after tetracosactide (Synacthen) 250mg im. Exclude Addisons if initial cortisol >l40nmoul/L. and 2nd cortisol >500nmol/L. Steroid drugs may interfere with this assay: check with local laboratory.

ACTH:
In Addisons disease ACTH >3Cong/L (iniappropriately high). Low in secondary causes.

Plasma reninactivity and aldosterone:

To assess mineralocortocoid status, Adrenal antibodies

AXR (plain abdominal films) and CXR:

Look for signs of previous TB, eg calcification. Have a low threshold far further investigations for TB, especially if autoantibodies are negative, eg CT adrenal glands.

Treatment:
Treat cause se Replace steroids: hydracortisone 20mg n morning, 10mg at bedtime po. Mineralocorticoid replacement may not be required: use blood pressure (postural hypotension), electrolytes and plasma renin activity (high if replacement required) as a guide. If necessary give fludrocortisone po 0.05mg every 2nd day to 0.15mg daily. Adjust on clinical grounds.

Addisons is often associated with other autoimmune diseases - even at the time of diagnosis. Hyperthyroidism should be looked for, particularly if there is an inadequate clinical response to treatment. Warm against abruptly discontinuing treatment Patients should have syringer at home (+ in-date parenteral hydrocortisone) for IM use in case vomiting prevents oral intake. Emphasize that any doctor or dentist giving treatment must know that the patient is taking steroids. Give steroid card.

Advise about wearing a bracelet declaring that steroids are taken. Must double steroids if febrile illness, injury, or severe stress. For dental treatment, double morning hydrocortisone. If vomiting, replace hydrocortisone with b .hydrocortisone sodium succinate 100mg IM, and seek medical help: IV fluids if fluids dehydrate .

Follow-up:
Yearly, including BP and U &E

Prognosis:
Normal lifespan if treated. Other causes of primary hypoadrenalism: Late-onset congenital adrenahyperplasia due to 2-hydroxylase deficiency, characterized by high urinary androgens